What is the primary treatment for a patient with a Ventricular Septal Defect (VSD)?

Management of Ventricular Septal Defects (VSDs)

Surgical closure is the primary treatment for patients with VSDs who have left ventricular volume overload and hemodynamically significant shunts (Qp:Qs ≥1.5:1), provided pulmonary artery systolic pressure is less than 50% systemic and pulmonary vascular resistance is less than one third systemic. 1

Indications for VSD Closure

Class I Indications (Strong Recommendation)

• VSDs with evidence of left ventricular volume overload and hemodynamically significant shunts (Qp:Qs ≥1.5:1) 1
• VSDs with Qp:Qs of 2.0 or more and clinical evidence of LV volume overload 1
• History of infective endocarditis caused by the VSD 1

Class IIa Indications (Reasonable to Perform)

• VSDs with net left-to-right shunting (Qp:Qs ≥1.5:1) with pulmonary artery pressure less than two-thirds systemic and pulmonary vascular resistance less than two-thirds systemic 1
• VSDs with net left-to-right shunting (Qp:Qs ≥1.5:1) in the presence of LV systolic or diastolic failure 1
• Perimembranous or supracristal VSDs with worsening aortic regurgitation 1

Class IIb Indications (May Be Considered)

• VSDs with a net left-to-right shunt (Qp:Qs ≥1.5:1) when pulmonary artery systolic pressure is 50% or more of systemic and/or pulmonary vascular resistance is greater than one-third systemic 1

Class III: Harm (Not Recommended)

• VSD closure should not be performed in adults with severe pulmonary arterial hypertension with pulmonary artery systolic pressure greater than two-thirds systemic, pulmonary vascular resistance greater than two-thirds systemic, and/or a net right-to-left shunt 1

Types of VSD and Closure Methods

Based on VSD Location

1. Perimembranous VSDs (80% of cases)

   • Surgical closure is recommended 2, 3
   • Percutaneous device closure is feasible but not recommended due to risk of complete heart block 3, 4

2. Supracristal VSDs (13% of cases)

   • Surgical closure is recommended, especially with aortic valve prolapse 2, 3

3. Muscular VSDs (4% of cases)

   • Percutaneous device closure is preferred beyond infancy 4
   • For infants: Hybrid approach or surgical closure on bypass 4
   • Amplatzer Muscular VSD Occluder is FDA-approved for muscular VSDs 5

4. Inlet VSDs (3% of cases)

   • Surgical closure is recommended 2

Surgical Considerations

• Surgery should be performed by surgeons with training and expertise in congenital heart disease 1
• Primary repair typically includes patch closure with synthetic material (Dacron, Gore-Tex) 1
• Careful intraoperative inspection with TEE is needed to rule out associated VSDs 1
• Mortality rate for surgical closure is very low (0.5%) 2
• Risk of complete heart block is minimal with surgical approach 2

Post-Procedure Follow-Up

• Adults with residual heart failure, shunts, pulmonary arterial hypertension, aortic regurgitation, or outflow tract obstruction should be seen at least annually at an adult congenital heart disease regional center 1
• Adults with a small residual VSD and no other lesions should be seen every 3-5 years 1
• Adults with device closure should be followed every 1-2 years depending on VSD location 1
• Adults with no residual VSD, no associated lesions, and normal pulmonary artery pressure do not require continued specialized follow-up 1

Complications to Monitor

• Development of complete heart block (rare with surgical approach) 2
• Residual shunts
• Aortic valve regurgitation (particularly with perimembranous and supracristal VSDs)
• Tricuspid valve regurgitation
• Infective endocarditis
• Arrhythmias

Special Considerations

• Pregnancy: Contraindicated in patients with VSD and Eisenmenger syndrome due to excessive maternal and fetal mortality 1
• Exercise: No restrictions for patients after VSD closure or with small VSDs without pulmonary hypertension. Patients with pulmonary arterial hypertension must limit themselves to low-intensity activities 1

Outcomes

Surgical closure of isolated VSDs has excellent outcomes with:

• Very low mortality (0.5%) 2
• Minimal risk of complete heart block 2
• Low reoperation rate (2.8% in immediate postoperative period) 2
• Excellent long-term results (99.5% of patients asymptomatic at follow-up) 2

The management of VSDs has evolved significantly, with excellent outcomes for both surgical and appropriate device closure approaches. The decision for intervention should be based on defect size, location, hemodynamic impact, and associated complications, with careful consideration of the contraindications in patients with severe pulmonary hypertension.